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Dysgammaglobulinemia

Dysgammaglobulinemias


Presentation

  • Presents outstanding photographs for virtually every common skin disorder.[books.google.com]
  • For the latter reason, the term immunoglobulin has been proposed to denote these proteins. 2 At the present time three major classes of immunoglobulins are recognized, designated gamma 2 , gamma 1 A and gamma 1 M.[nejm.org]
  • 0 Shares We present a pediatric case of recurrent optic neuritis, celiac disease, partial IgA and IgG3 deficiency in the context of anti-aquaporin-4 auto-immunity and familial IgA deficiency with celiac disease.[guthyjacksonfoundation.org]
  • Abstract A family with type I dysgammaglobulinemia is presented in which a brother and sister were affected.[annals.org]
  • The preponderance of males among recognized examples of dysgammaglobulinemia, and the presently reported patient whose male sibling also appears to have had a congenital gamma globulin deficiency, suggest that dysgammaglobulinemia, in this particular[pediatrics.aappublications.org]
Myelopathy
  • Journal of Clinical Neuro-Ophthalmology: December 1991 Articles: PDF Only Abstract At the age of 12, a prematurely born boy with an otherwise unremarkable past medical history developed bilateral optic neuritis associated with transverse myelopathy.[journals.lww.com]
  • Deficiency; Immunoglobulins, Intravenous; Male; Optic Neuritis; Prednisone; Visual Acuity Abstract At the age of 12, a prematurely born boy with an otherwise unremarkable past medical history developed bilateral optic neuritis associated with transverse myelopathy[collections.lib.utah.edu]
Chorea
  • […] subjects (n 22) by enzyme-linked immunosorbent assay (ELISA) for the levels of total and specific IgG, IgM, and IgA against antigens previously identified in multiple sclerosis (myelin basic protein and myelin-associated glycoprotein) and Sydenham's chorea[ncbi.nlm.nih.gov]

Workup

  • A: I believe that your workup is sufficient. Copied below, for your interest, is a posting from our “Ask the Expert” website in response to a similar inquiry. This will give you references to pursue in regards to your question.[aaaai.org]

Treatment

  • Spiramycin was added toward the end of the treatment period. This patient failed to show clinical response although there was a decrease in the stool oocyst count from the value just prior to therapy.[ncbi.nlm.nih.gov]
  • This service should be used for second opinions, additional medical reviews and consultations; not for primary diagnosis or treatment.[xmri.com]
  • Treatment with tacrolimus was successful in preventing disease relapses. This case stresses the relevance of central nervous system anti-aquaporin-4 auto-immunity in a broader context of immune dysregulation and neuro-immunology.[guthyjacksonfoundation.org]
  • Treatment: Selective IgA deficiency has no known cure. Treatment aims to control symptoms of associated diseases.[med-help.net]
  • Features extensive revisions throughout that highlight the newest developments in diagnosis and treatment, giving you the absolute latest on virtually every skin disorder.[books.google.com]

Prognosis

  • Prognosis - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Treatment - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Resources - Low birth weight dwarfism dysgammaglobulinemia Not supplied.[checkorphan.org]
  • The prognosis has been poor, with little efficacy for corticosteroids, splenectomy, or thymectomy.[neutropenia.ca]
  • The prognosis is similarly poor for XLP2 without HCT, in which mortality is attributed to HLH (30%), colitis (23%), liver failure (8%), and infectious causes (8%) (Pachlopnik Schmid et al., 2011).[atlasgeneticsoncology.org]

Etiology

  • It is obvious that more knowledge of etiologic factors and pathogenetic mechanisms is necessary to classify, cure, and eventually prevent the diseases described in this paper.[ncbi.nlm.nih.gov]
  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • Viral culture or examintion of vesicle contents by indirect immunofluorescent technic can provide definitive diagnosis of the etiology of a zosteriform eruption. Clin Infect Dis . 2010 Jul 15;51(2):197-213.[aaaai.org]
  • Suggested etiologies include rapid isotype switching of B cells from production of IgM to production of other isotypes and hypercatabolism of IgM.[emedicine.medscape.com]

Epidemiology

  • Epidemiology Frequency United States In a retrospective study of a large allergy practice (20,000 patients) database, Goldstein et al reported prevalences of SIgM deficiency of 0.26% among adults and 0.03% among children. [3, 4 ] International SIgM deficiency[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Although the book’s primary focus is on practical diagnosis and management, the pathophysiology of PID is also discussed.[books.google.com]
  • Myelokathexis Intramedullary destruction of neutrophils appears to be the pathophysiologic basis of myelokathexis. Patients demonstrate moderate neutropenia with morphologic abnormality of the neutrophilic nuclei.[neutropenia.ca]
  • This reflects major differences in the pathophysiology of latency and reactivation of these 2 alphaherpesviruses.[aaaai.org]
  • Pathophysiology The cause of SIgM deficiency is unknown.[emedicine.medscape.com]

Prevention

  • It is obvious that more knowledge of etiologic factors and pathogenetic mechanisms is necessary to classify, cure, and eventually prevent the diseases described in this paper.[ncbi.nlm.nih.gov]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • Treatment with tacrolimus was successful in preventing disease relapses. This case stresses the relevance of central nervous system anti-aquaporin-4 auto-immunity in a broader context of immune dysregulation and neuro-immunology.[guthyjacksonfoundation.org]
  • One form results in a decrease in the rate of synthesis of specific heavy chains (alpha and gamma) and a compensatory increase of competent IgM, thus usually preventing an antibody deficiency syndrome.[annals.org]
  • Prevention - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Diagnosis - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Prognosis - Low birth weight dwarfism dysgammaglobulinemia Not supplied.[checkorphan.org]

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